We investigated the impact of Bazett (B) and Fridericia (F) correction formulas on the evaluation of QT prolongation in patients during methadone administration and to explore the relationship between heart rate and corrected QT interval (QTc) according to both correction formulas. This study was conducted as a single-center, retrospective observational study. Subjects were cancer patients who underwent electrocardiographic evaluation for methadone therapy at our institute from April 1, 2013, to August 31, 2023. The study assessed the incidence of QT prolongation and analyzed the correlation between heart rate and QTc derived from the B and F formulas. The mean QTc of 430.3±25.8 msec for the B formula and 409.2±20.8 msec for the F formula. The incidence of QT prolongation was significantly lower with the F formula (8.4%) compared to the B formula (27.7%), indicating a 19.3% reduction in QT prolongation cases (p<0.001). Additionally, the difference in QTc between the two formulas increased with an increase in heart rate (p<0.001). These results suggest that the F formula for QT interval correction in methadone therapy potentially expands the eligible patients for this therapy.

Congenital myotonic dystrophy is the most severe form of inherited myotonic dystrophy, presenting with generalized muscle weakness, breathing problems, and feeding difficulties. We report a patient with congenital myotonic dystrophy who began rehabilitation early in the neonatal intensive care unit. The mother had myotonic dystrophy and experienced no complications during pregnancy. The patient was born at a gestational age of 38 weeks and 1 day, weighing 2712 g, with an Apgar score of 1/4, and was diagnosed with congenital myotonic dystrophy. Marked generalized hypotonia and respiratory compromise were observed from birth, and the patient was orally intubated and managed with a ventilator. Rehabilitation to promote sensory-motor development was started 13 days after birth. Initially, generalized muscle hypotonia and prominent limitation of joint range of motion were observed. Therefore, joint range of motion exercises, positioning, and rehabilitation of the sensory-motor system were conducted first, while monitoring circulatory and respiratory status. After weaning from the ventilator, feeding evaluation was started on the 123rd day of life. In collaboration with physicians and nurses, feeding volume was increased stepwise to safely introduce oral feeding. The infant gradually became capable of oral intake and was discharged from the hospital on the 160th day of life. In this report, we demonstrated that rehabilitation intervention for infants with congenital myotonic dystrophy from the time of admission to the neonatal intensive care unit contributed to improved motor development, initiation of oral feeding, and smooth discharge.

Congenital myotonic dystrophy is the most severe form of inherited myotonic dystrophy, presenting with generalized muscle weakness, breathing problems, and feeding difficulties. We report a patient with congenital myotonic dystrophy who began rehabilitation early in the neonatal intensive care unit. The mother had myotonic dystrophy and experienced no complications during pregnancy. The patient was born at a gestational age of 38 weeks and 1 day, weighing 2712 g, with an Apgar score of 1/4, and was diagnosed with congenital myotonic dystrophy. Marked generalized hypotonia and respiratory compromise were observed from birth, and the patient was orally intubated and managed with a ventilator. Rehabilitation to promote sensory-motor development was started 13 days after birth. Initially, generalized muscle hypotonia and prominent limitation of joint range of motion were observed. Therefore, joint range of motion exercises, positioning, and rehabilitation of the sensory-motor system were conducted first, while monitoring circulatory and respiratory status. After weaning from the ventilator, feeding evaluation was started on the 123rd day of life. In collaboration with physicians and nurses, feeding volume was increased stepwise to safely introduce oral feeding. The infant gradually became capable of oral intake and was discharged from the hospital on the 160th day of life. In this report, we demonstrated that rehabilitation intervention for infants with congenital myotonic dystrophy from the time of admission to the neonatal intensive care unit contributed to improved motor development, initiation of oral feeding, and smooth discharge.

Spinal muscular atrophy is a neuromuscular disease characterized by muscle atrophy and progressive muscle weakness due to the degeneration of motor neurons in the anterior horn of the spinal cord. We report a case of an adult patient with spinal muscular atrophy type II and difficulty holding a sitting position. The patient was evaluated before and after Nusinersen treatment and thereafter periodically for up to 3 months for motor and daily living functions. At 3 months post-treatment, the Expanded version of the Hammersmith Functional Motor Scale and the Revised Upper Limb Module, which are motor function assessment tools for evaluating spinal muscular atrophy, showed an increase of 2 points. Evaluation of daily functioning using the Canadian occupational performance measure demonstrated improvements in eating and computer finger manipulation, and these improvements were considered important in daily lives by the patient. This report shows that the Nusinersen treatment improved motor and daily life functions in a patient with spinal muscular atrophy and low motor function. The report also concludes that rehabilitation evaluation for spinal muscular atrophy should include a disease-specific assessment of motor function, combined with an assessment focusing on physical symptoms and daily life functions to capture clinical changes that are responsive to individual patients with spinal muscular atrophy.

Spinal muscular atrophy is a neuromuscular disease characterized by muscle atrophy and progressive muscle weakness due to the degeneration of motor neurons in the anterior horn of the spinal cord. We report a case of an adult patient with spinal muscular atrophy type II and difficulty holding a sitting position. The patient was evaluated before and after Nusinersen treatment and thereafter periodically for up to 3 months for motor and daily living functions. At 3 months post-treatment, the Expanded version of the Hammersmith Functional Motor Scale and the Revised Upper Limb Module, which are motor function assessment tools for evaluating spinal muscular atrophy, showed an increase of 2 points. Evaluation of daily functioning using the Canadian occupational performance measure demonstrated improvements in eating and computer finger manipulation, and these improvements were considered important in daily lives by the patient. This report shows that the Nusinersen treatment improved motor and daily life functions in a patient with spinal muscular atrophy and low motor function. The report also concludes that rehabilitation evaluation for spinal muscular atrophy should include a disease-specific assessment of motor function, combined with an assessment focusing on physical symptoms and daily life functions to capture clinical changes that are responsive to individual patients with spinal muscular atrophy.

Wolf-Hirschhorn syndrome is a chromosomal aberration caused by a deletion of the distal short arm of chromosome 4, characterized by distinct craniofacial features, failure to thrive, psychomotor developmental retardation, epilepsy, and feeding disorders. We report a case of patient with Wolf-Hirschhorn syndrome who underwent interventional rehabilitation commencing from the neonatal period in the neonatal intensive care unit. The patient was born at gestational age of 38 weeks 0 days, weighing 1583 g, with an Apgar score of 4/9, and was diagnosed with partial monosomy of the short arm of chromosome 4. Postnatal inspiratory stridor exacerbation was noted for which high-flow nasal cannula therapy was initiated. Rehabilitation commenced on the 18th day after the infant's birth, to promote sensorimotor development. Initially, the trunk was in a low muscle tension and unstable state. Therefore, we first prescribed rest followed by sensorimotor rehabilitation. When the infant's clinical condition stabilized, we performed prone and anti-gravity hugging exercises to improve the low trunk tension. Breastfeeding evaluation began 56 days after birth, when the respiratory condition improved. We practiced feeding the infant orally, in collaboration with doctors and nurses, to reduce bending and stabilize the posture when raising the mandible. The infant was gradually able to feed orally and gained weight. Thereafter, he was discharged 141 days after birth. This report concluded that rehabilitation intervention from the neonatal period, in collaboration with the multidisciplinary team and patient's family, contributed to initiation of oral feeding, improvement of sensorimotor development, and smooth transition to home care.

Wolf-Hirschhorn syndrome is a chromosomal aberration caused by a deletion of the distal short arm of chromosome 4, characterized by distinct craniofacial features, failure to thrive, psychomotor developmental retardation, epilepsy, and feeding disorders. We report a case of patient with Wolf-Hirschhorn syndrome who underwent interventional rehabilitation commencing from the neonatal period in the neonatal intensive care unit. The patient was born at gestational age of 38 weeks 0 days, weighing 1583 g, with an Apgar score of 4/9, and was diagnosed with partial monosomy of the short arm of chromosome 4. Postnatal inspiratory stridor exacerbation was noted for which high-flow nasal cannula therapy was initiated. Rehabilitation commenced on the 18th day after the infant's birth, to promote sensorimotor development. Initially, the trunk was in a low muscle tension and unstable state. Therefore, we first prescribed rest followed by sensorimotor rehabilitation. When the infant's clinical condition stabilized, we performed prone and anti-gravity hugging exercises to improve the low trunk tension. Breastfeeding evaluation began 56 days after birth, when the respiratory condition improved. We practiced feeding the infant orally, in collaboration with doctors and nurses, to reduce bending and stabilize the posture when raising the mandible. The infant was gradually able to feed orally and gained weight. Thereafter, he was discharged 141 days after birth. This report concluded that rehabilitation intervention from the neonatal period, in collaboration with the multidisciplinary team and patient's family, contributed to initiation of oral feeding, improvement of sensorimotor development, and smooth transition to home care.

BACKGROUND: Systemic and local factors may lead to disruption of craniofacial growth and development, causing an imbalance between the orofacial skeleton, muscle and soft tissue, dental occlusion, and the dental arch during growth periods. We aimed to reveal whether the prevalence of incompetent lip seal (ILS) varies with age and region, as well as to clarify the factors related to an ILS, in a national, large-scale epidemiological study. METHODS: We surveyed 3399 children, from 3 to 12 years of age, visiting 66 pediatric dental clinics throughout Japan. For this survey, we employed a questionnaire consisting of 44 questions regarding daily health conditions and lifestyle habits. We evaluated the differences in ILS prevalence by age and region (using a Cochran-Armitage test for trend and a Kruskal-Wallis test), and the relationship between ILS and factors investigated in the questionnaire (using Spearman's rank correlation coefficient). RESULTS: We observed that 30.7% of Japanese children exhibited an ILS and that the ILS rate increased with age (p < 0.001). There were no regional differences in the rate of ILS in Japanese children (p = 0.506). We revealed that 12 of 44 survey items exhibited a statistically significant correlation with ILS (p < 0.001), using Spearman's rank correlation coefficient. These items involved orofacial morphology, mouth breathing, and possibly, allergic rhinitis. CONCLUSION The rate of ILS seems to increase with age in children, throughout Japan. Therefore, this disorder may not self-correct during the growth periods in these children. Guidelines are required for pediatric dentists to recognize ILS among children aged 3-12 years.
Child ; Child, Preschool ; Cross-Sectional Studies ; Female ; Humans ; Japan/epidemiology* ; Lip/abnormalities* ; Male ; Prevalence

BACKGROUND: It has been pointed out that prolonged television (TV) viewing is one of the sedentary behaviors that is harmful to health; however, the association between socioeconomic status (SES) and prolonged TV viewing time has not been sufficiently investigated in Japan. METHODS: The study population are the participants of NIPPON DATA2010, which is a prospective cohort study of the National Health and Nutrition Survey 2010 in Japan. They were residents in 300 randomly selected areas across Japan. This study included 2752 adults. SES was classified according to the employment status, educational attainment, living status, and equivalent household expenditure (EHE). Prolonged TV viewing time was defined as more than or equal to 4 h of TV viewing per day. Multivariable logistic regression analyses were conducted to examine the association of SES with prolonged TV viewing time. RESULTS: The mean TV viewing time was 2.92 h in all participants. Of 2752 participants, 809 (29.4%) prolonged TV viewing, and the mean TV viewing time of them was 5.61 h. The mean TV viewing time in participants without prolonged TV viewing time was 1.81 h. The mean TV viewing time was prolonged as age classes increased and significantly longer in aged ≥60 years. Prolonged TV viewing time was associated with not working for all age classes and sexes. Only among women, education attainment and living status were also associated with prolonged TV viewing time. For education attainment, the lower the received years of education, the higher odds ratios (OR) of prolonged TV viewing time. For living status, in women aged <60 years, living with others had a significantly higher OR compared to living with spouse. On the other hand, in women aged ≥60 years, living alone had a significantly higher OR. EHE did not have any significant associations with prolonged TV viewing time. CONCLUSIONS In a general Japanese population, it should be noted that the association between SES and prolonged TV viewing time differed by age and sex. Particularly, it must draw attention to the prolonged TV viewing in elderly. The intervention in order to shorten TV viewing time needs to consider these attributes.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Educational Status ; Female ; Humans ; Infant ; Japan ; Male ; Middle Aged ; Prospective Studies ; Sedentary Behavior ; Sex Factors ; Social Class ; Socioeconomic Factors ; Television/statistics & numerical data* ; Young Adult